Amyloidosis - cerebral

Definition

Senile cerebral amyloid angiopathy (CAA) is neurological condition in which amyloid protein is deposited onto the walls of the arteries of the brain. CAA increases the risk of bleeding into the brain (hemorrhagic stroke).

Alternative Names

Causes

Senile cerebral amyloid angiopathy is a cause of lobar intracerebral hemorrhage (bleeding in a localized area of the brain, a form of stroke) in the elderly. There may be multiple episodes of bleeding, which may occur over a period of many months. The symptoms occur because bleeding in the brain harms brain tissue.

The cause is unknown. Amyloid protein is deposited in the arterial walls of the brain, and there are often no deposits elsewhere in the body. The major risk factor is age. The incidence is much greater in people older than 60.

Symptoms

• Headache (usually focal, localized to a specific part of the head)
• Vomiting
• Dementia (loss of cognitive ability)
• Drowsiness
• Neurologic changes (variable)
  • May be sudden in onset
  • Double vision, decreased vision
  • Speech difficulties
  • Confusion, delirium
  • Weakness or paralysis
  • Sensation changes or loss of sensation in an area
• Seizures
• Stupor or coma (rarely)

Exams and Tests

An examination shows focal neurologic deficits (brain function changes) that reflect the location of the bleeding. If there is a history of dementia, lobar hemorrhage may be suspected.

A CT scan or MRI of the head shows lobar intracerebral hemorrhage. Often, MRI or CT scan will show evidence of prior bleeding episodes that may or may not have been noticed. An angiography of the brain may be used to rule out arteriovenous malformation

Senile cerebral amyloid angiopathy is not specifically diagnosed until after death, when a postmortem biopsy reveals amyloid deposits in the blood vessels of the brain.

Treatment

There is no known effective treatment. Treatment is supportive and based on the control of symptoms. In some cases, rehabilitation is needed for weakness or clumsiness. This can include physical, occupational, or speech therapy.

Occasionally, some patients are good candidates for medications that can help improve memory.

Outlook (Prognosis)

The disorder is generally progressive. It cannot be definitively diagnosed in most cases until after death.

Possible Complications

• Lobar intracerebral hemorrhage, repeated episodes
• Dementia
• Seizures
• Hydrocephalus ("water on the brain") (rarely)

When to Contact a Medical Professional

Go to the emergency room or call the local emergency number (such as 911) if there is any sudden loss of movement, sensation, vision, speech or other impairments that may indicate a loss of neurologic functions.